Cushing's disease is a disease of the endocrine system that develops as a result of damage to the hypothalamic-pituitary system and increased production of the hormone ACTH by the adrenal cortex. This pathology is characterized by multisymptomaticity. Most often, the disease develops in women. Obesity, arterial hypertension, diabetes mellitus and many other specific manifestations are diagnosed in patients suffering from Itsenko-Cushing's disease.
To confirm the assumption of this disease, the patient will have to undergo a series of biochemical blood tests, hormone tests, X-rays of the spine and chest, CT of the pituitary and adrenal glands. Pathology is treated with medication, radiation and surgery.
What is this disease
In medicine, there are two concepts: disease and Itsenko-Cushing's syndrome. In the second case, we are talking about the primary manifestations of hypercortisolism. Anddisease, and Itsenko-Cushing's syndrome have the same symptom complex, but at the same time have a different nature of origin.
The development of the syndrome is based on the increased production of the human hormone - glucocorticosteroid, which is caused by hyperplastic tumors of the adrenal cortex. Basically, such a malfunction in the endocrine system occurs due to an increasing corticosteroma, glucosteroma, adenocarcinoma. The cause of Itsengo-Cushing's syndrome can also be hypercortisolism, provoked by long-term use of synthetic steroids. In rare cases, the prerequisites for the development of this pathology are cancerous tumors of the ovary, lungs, pancreas, intestines, and thyroid gland. Against the background of these malignant processes, an ectopic ACTH syndrome develops with similar clinical manifestations.
Unlike the syndrome, Cushing's disease originates at the level of the hypothalamic-pituitary system, and the adrenal glands and other glands of the endocrine system are involved in the pathological process at the later stages. The disease occurs in the fairer sex several times more often than in men, while most often the disease affects women of childbearing age. The course of the disease is characterized by severe neuroendocrine disorders, including metabolic disorders, neurological symptoms, malfunctions of the cardiovascular, digestive and excretory systems.
Provoking factors
The causes of Cushing's disease lie in the presence of a pituitary adenoma that producesexcessive amount of adrenocorticotropic hormone. In the case of a tumor lesion of this gland of the brain, patients have adenocarcinoma, micro- or macroadenoma. Often, the occurrence of these neoplasms is associated with the consequences of past infections and damage to the central nervous system (encephalitis, arachnoiditis, meningitis), craniocerebral trauma, and intoxication. In medical practice, there have been cases when Cushing's disease in women developed against the background of hormonal disorders caused by pregnancy, childbirth, menopause.
Pathogenesis
The mechanism of the development of Itsenko-Cushing's pathology is a violation of the hypothalamic-pituitary-adrenal relationship, as a result of which too much ACT of the hormone is produced. In turn, the increased synthesis of this substance serves as an explanation for a whole string of adrenal and extra-adrenal reactions in the body:
- In the adrenal glands, the synthesis of glucocorticoids and male hormones increases, while the secretion of mineralocorticoids decreases.
- Excessive amount of glucocorticoids negatively affects protein-carbohydrate metabolism, which leads to connective tissue atrophy, hyperglycemia and insulin resistance, preceding the development of hormonal diabetes mellitus.
- Incorrect fat metabolism is a favorable condition for the development of obesity.
- Hypokalemia and high blood pressure are a consequence of reduced mineralconticoid activity.
- The catabolic effect of hormones on bone tissue prevents the full absorption of calcium inGastrointestinal tract, so osteoporosis develops.
- Increased production of androgens (male hormones) causes ovarian dysfunction and disruption of the female reproductive system.
Degrees of severity
There are several forms of the clinical course of Itsenko-Cushing's disease. The disease has three degrees of severity:
- Easy. The disease is accompanied by mild symptoms: the menstrual cycle can be preserved, and signs of osteoporosis can be completely absent.
- Average. Symptoms of Cushing's disease are almost impossible to confuse with manifestations of other diseases, but at this stage the disease does not progress, the general condition of the patient remains satisfactory.
- Heavy. This form of pathology is characterized by the development of muscle atrophy, hypokalemia, arterial hypertension, mental disorders.
The course of the disease is also distinguished by the rate of increase of pathological changes - a progressive and torpid form of the disease is distinguished. In the first case, the disease develops over 6-12 months. The changes that occur with the patient do not go unnoticed by others. With a torpid course, the signs of the disease are formed in stages, over 3-10 years.
How appearance changes
Recognizing the symptoms of Itsenko-Cushing's disease will not be difficult even for those who are far from medicine. To do this, you need to know what disorders occur with this disease and how the appearance of a person with this disease changes along with them.
Patients,those suffering from the disease are distinguished by the so-called Cushingoid appearance, which is characterized by:
- specific deposition of adipose tissue in typical areas - on the face, shoulders, neck, chest, back, abdomen;
- as the disease progresses, the face becomes moon-shaped;
- a fatty hump forms in the region of the VI-VII cervical vertebrae;
- the chest and abdomen increase in volume, and the limbs remain relatively thin;
- skin can peel off, become dry, while a pronounced purple-marble pattern will be noticeable on it;
- stretch marks and subcutaneous hemorrhages are observed in the area of the mammary glands, shoulders and lower abdomen;
- Patients with Cushing's disease often suffer from acne and furunculosis.
Other signs and symptoms
In women with Itsenko-Cushing's syndrome, the menstrual cycle fails, in some cases, menstruation completely disappears. In addition, it is impossible not to note the excessive atypical male pattern hair growth - this phenomenon in medicine is called "hirsutism". We are talking about vegetation on the face, neck, chin, temples, abdomen, chest and intense hair loss on the head.
In men suffering from Cushing's disease, on the contrary, hair falls out on the body, the growth of stubble on the face stops. Over time, in patients, regardless of gender, libido decreases, sexual desire is lost. If the disease strikes in childhood,it is likely that sexual development and growth will be delayed due to a decrease in the secretion of gonadotropic hormone.
Due to calcium reabsorption and the development of osteoporosis, bone deformity occurs, fractures and joint pain are possible. Over time, tachycardia, cardiopathy and chronic heart failure are added to arterial hypertension. Patients in this category are more likely to suffer from bronchitis, pneumonia and tuberculosis.
It is impossible to ignore the damage to the digestive system in the development of Itsenko-Cushing's syndrome. Patients often complain of increased heartburn, pain in the epigastric region caused by "steroid" peptic ulcer and bleeding within the gastrointestinal tract. Possible disorders in the functioning of the kidneys, lesions of the urinary tract up to uremia are not excluded.
Neurological problems
The progression of Itsenko-Cushing's pathology threatens the patient with the development of pain, amyotrophic, stem-cerebellar and pyramidal syndromes. But if the pain and amyotrophic syndromes are amenable to drug control, then the changes caused by the stem-cerebellar and pyramidal syndromes cannot be contained, they are irreversible. Patients may experience ataxia, nystagmus, and abnormal reflexes involving the facial and hypoglossal nerves.
In Itsenko-Cushing's disease, psycho-emotional disorders often occur on the basis of neurasthenic, depressive, astheno-adynamic and epileptiform syndromes. A completely natural phenomenon is the deterioration of memory and intelligence, lethargy, a decrease in the amplitudeemotional fluctuations. Some patients have obsessive suicidal thoughts.
Cushing's disease in dogs
Interestingly, this disease is diagnosed in quadrupeds as often as in humans. The cause of the disease is a similar effect of cortisol on the work of the whole organism. In an animal with Itsenko-Cushing syndrome, the immune system, nervous and genitourinary systems are affected, the heart suffers, the condition of the connective tissue and internal organs worsens. The disease almost never develops rapidly, so pet owners have enough time and good chances to help the animal and prolong its life. The following symptoms should alert:
- increased thirst;
- frequent urination;
- exorbitant appetite;
- deterioration of the condition of the coat, the appearance of symmetrical bald patches.
You can also observe other signs of Cushing's disease. In dogs, along with increased urge to urinate, urinary incontinence appears. The pet is gaining excess weight, and fat deposits are localized mainly in the neck, abdomen and chest. Over time, weakened muscles become unable to hold an enlarged abdomen, which makes it sag. The dog becomes inactive, lethargic, drowsy, possibly unbalanced behavior. If the cause of the disease was a pituitary adenoma, there are violations of coordination.
Examination and diagnostics
To start treatment, it is not enough just to complain of the patient and see a doctor. For stagingdiagnosis, consultations with an endocrinologist, neuropathologist, gastroenterologist, cardiologist and gynecologist will be required.
Specialists in their arguments rely on the results of biochemical blood tests - a number of indicators undergo typical changes in Cushing's disease. Diagnosis allows us to conclude that there is an increased content of cholesterol, chlorine and sodium in the blood. In patients with this disease, the level of sugar in the blood and urine significantly exceeds the norm, which indicates the development of a characteristic "steroid" diabetes mellitus.
A mandatory blood test for hormones, which allows you to detect the concentration of cortisol, adrenocorticotropic hormone, renin. The cortisol content is also taken into account in the patient's urine.
In addition to laboratory research methods, the patient must undergo:
- X-ray of the skull. Particular attention is paid to the study of the Turkish saddle - the location of the pituitary gland. A macroadenoma can be seen on an x-ray.
- X-ray of the spine. Performed to detect signs of osteoporosis.
- CT and MRI of the brain with the introduction of gadolinium (contrast agent). During diagnostic procedures, macro- and microadenomas are detected.
- Ultrasound of the adrenal glands. In Cushing's disease, bilateral organ hyperplasia is diagnosed. At the same time, an increase in the adrenal gland on only one side is often regarded as the formation of a glucosteroma.
Therapy and surgery
Since Itsenko-Cushing's disease develops against the background of impaired ACTH secretion, treatmentimplies the elimination of endocrine disorders and the restoration of metabolic processes. The therapeutic program involves the use of medications, radiotherapy of the hypothalamic-pituitary region. In severe cases, the most effective treatment option is surgery or a combination of treatments.
Medicines are prescribed to patients only in the early stages of the disease. Blockade of pituitary functions is an intermediate goal of drug exposure. To achieve it, drugs such as Reserpine, Bromocriptine are prescribed - they actively suppress the secretion of ACTH. In addition to centrally acting hormones, blockers of the synthesis of adrenal steroids can be used in the treatment of Itsenko-Cushing's disease. Symptomatic therapy is mandatory, correcting protein, mineral, carbohydrate, and electrolyte metabolism.
Radiation therapy is often given to patients who do not have x-ray evidence of a pituitary tumor. One of the methods of treatment is the implantation of radioactive isotopes into the pituitary gland. A few months after therapy, a period of remission begins, which is accompanied by a decrease in body weight, blood pressure, stabilization of the menstrual cycle, inhibition of the pathological process and damage to bone and muscle tissues. Irradiation of the pituitary gland is often combined with unilateral removal of the adrenal gland (adrenalectomy).
In severe Cushing's disease, the treatment is the removal of the adrenal glands from both sides(bilateral adrenalectomy), which further requires lifelong replacement therapy with glucocorticosteroids and mineralocorticoids.
If the cause of the disease is a benign pituitary tumor, endoscopic transnasal or transcranial removal of the adenoma is performed. In some cases, the operation to excise the affected part of the pituitary gland is performed by the transsphenoidal route. After removal of the adenoma, remission occurs in the majority of cases, but approximately one in five patients relapse.
What is the forecast
Determining the chances of a person with Cushing's disease recovering is not easy. The outcome of the disease depends on the severity of the disease, the age of the patient. With timely treatment of pathology at an early stage in young patients, a complete recovery is not excluded. While the long course of Cushing's disease leads to persistent changes in the bone tissue, cardiovascular and genitourinary systems, negatively affects the ability to work and aggravates the prognosis, even if the underlying cause is subsequently eliminated. Advanced forms of this disease end in death due to the addition of infections, septic complications, and the development of renal failure.
Patients with Itsenko-Cushing pathology should be regularly observed by an endocrinologist, cardiologist, neurologist, gynecologist. In order to prevent the progression of the disease, it is important to avoid exorbitant physical and psycho-emotional stress, treat infectious diseases, intoxications, and notallow complications. With such a diagnosis, work activity on night shifts is completely excluded.
